PDF(Pubmed)
Abstract：
Osteosclerosis in multiple myeloma (MM) is typically associated with rare POEMS syndrome, characterized by polyneuropathy (P), organomegaly (O), endocrinopathy (E), M-protein (M), and skin changes (S). However, osteosclerosis in multiple myeloma (MM) without POEMS syndrome, defined as non-POEMS Osteosclerotic MM, is exceedingly rare. We report a 70-year-old man with rib pain, remarkably high bone mineral density and diffuse osteosclerosis. The diagnosis of non-POEMS osteosclerotic MM was confirmed by biopsy and aspiration of bone marrow through surgery. A literature review spanning from 1990 identified 12 cases of similar non-POEMS osteosclerotic MM, including 5 males and 7 females with a mean age of 59.7 ± 10.6 years. The non-POEMS osteosclerotic MM can be divided into two subtypes, the osteosclerotic lesion subtype and the diffuse osteosclerosis subtype. Absence of polyneuropathy and organomegaly are the main factors that differentiate non-POEMS osteosclerotic MM from POEMS. A hyperactive osteoblastic process might be the etiology of diffuse osteosclerosis. Further research is needed to understand its etiology and pathophysiology.
摘要：
多发性骨髓瘤（MM）的骨硬化通常与罕见的POEMS综合征有关，以多发性神经病（P）为特征，器官肿大（O），内分泌病（E），M-蛋白(M),和皮肤变化（S）。然而,多发性骨髓瘤（MM）无POEMS综合征的骨硬化，定义为非POEMS骨硬化性MM，非常罕见。我们报道了一位70岁的肋骨疼痛患者,非常高的骨密度和弥漫性骨硬化。通过活检和手术抽吸骨髓证实了非POEMS骨硬化性MM的诊断。从1990年开始的文献综述确定了12例类似的非POEMS骨硬化性MM，包括5名男性和7名女性，平均年龄为59.7±10.6岁。非POEMS骨硬化性MM可分为两种亚型，骨硬化病变亚型和弥漫性骨硬化亚型。缺乏多发性神经病和器官肿大是区分非POEMS骨硬化性MM和POEMS的主要因素。过度活跃的成骨细胞过程可能是弥漫性骨硬化的病因。需要进一步研究以了解其病因和病理生理学。
